"Suffering the Slings and Arrows of Outrageous Fortune"

Posts tagged ‘The Driscoll Theory’

Since February 9th, Em has had 75 episodes that are seizure-like. Sometimes she has several a day. Sometimes she goes a few days between episodes.

They look like a seizure but certainly not exactly. Now, it is possible that some of these episodes could be a type of seizure and the upcoming EEG monitoring should help us figure that out. But, I got a suggestion that tipped me off to what I think is a more likely possibility and I am very VERY grateful to Paulette for suggesting it because I suspect it would have been a very long time before the doctors would have figured it out.

Complex Migraine (or Migraine with Aura) is what I am now convinced this is. Apparently, the aura of a migraine can look very much like a seizure or even a stroke. I think we didn’t notice the headache that typically follows the episodes because, let’s be honest, she pretty much always has a headache. Even when she complained that her headache was horrible afterwards – well, not only does she always have a headache, but headaches often follow a seizure, so it seemed to make sense. But, after doing some research, it makes more sense that this is a Migraine with Aura. Furthermore, I am wondering if she is specifically having basilar-type migraines, which would fall under the umbrella of Complex Migraine.

Initially, I was convinced the episodes were triggered by cervical instability – the first episode was in the car while she was wearing her hard collar because her neck was so unstable. She complained of her vision being “weird”, then was unable to move or talk and was in and out of consciousness – I thought she had just fallen asleep. She was barely able to walk once we got home 30 minutes later, then the symptoms resolved completely and she was fine. (Well, not fine, but the point is the symptoms wholly resolved until the next episode.) By the 4th episode, my records show her describing feeling like her head was “sitting on a burning spike”. Another time, she described the pain like her head was “expanding like a balloon when she breathes”. Looking back, she says she almost always had a worse headache after these episodes.

The length of a migraine aura is 5 to 60 minutes, while seizures typically last 2 or 3 minutes. Her episodes tend to last between 5 and 30 minutes – the longest being far too long for a seizure but right in the range of a complex migraine. In the early episodes, she tended to go limp for several minutes. Eventually, the episodes started looking more like a tonic clonic seizure – but not quite as violent. Just a lot of twitching and jerking and twisting stiffly and then going limp, often repeatedly. She often has trouble speaking, swallowing, and breathing during the episodes. Sometimes her eyes are closed, sometimes her eyes are open and dart back and forth the whole time. She sometimes has a black horizontal line through her visual field that eventually fades. She often can’t move her legs for several minutes after. Often, she is aware during, but goes in and out of consciousness. She is dazed, confused, and dizzy for 5 to 15 minutes after.

The headache part is hard because she has such layers of headaches. She has been dealing with high pressure headaches, then low pressure after the LP, then bouncing back and forth. So it has been hard to sift through all of that. But, she has been having what we now believe are legit migraines and they mostly follow these episodes, although she has had a few without the aura. They don’t respond to Diamox, she is very sensitive to light and sound, she is nauseated and the headache eventually passes a few hours later. She sometimes has multiple episodes in a day that result in the headache never going away and just getting worse with each episode. The headache is bilateral and originates at the base of her skull and wraps around to her temples. (These points are what makes me suspect a basilar-type migraine. If I am right, then my initial suspicion of brain stem involvement wasn’t entirely wrong.)

I have been keeping detailed records of the episodes but it finally occurred to me to keep track of her headache pain before and after the episodes. Typically, her headache is a 2 or 3 before and a 6 or 7 immediately after, increasing to an 8 or 9 within a few minutes.

These episodes are now mostly triggered by flashing lights: police lights, strobe lights, sunlight flickering through trees while driving, etc. There is one stretch of road on our way to Speech Therapy that is all woods – if it is a sunny day, she is almost guaranteed an episode. Watching TV shows with flashing lights on NetFlix often ends up triggering an episode. One episode was started by police lights that went by our house. (I never, ever realized how many flashing lights are in this world until now. It is ridiculous!) Fluorescent lighting is problematic, although I don’t think she has ever had an episode specifically triggered by it. She can sometimes avoid an episode by avoiding flashing lights: when driving or watching TV, she can avert or close her eyes and not go there. Not fool proof, but she does have some control. Sleep deprivation also seems to trigger them and sometimes they just happen with no obvious trigger.

The concussion from December probably has a lot to do with these episodes, in my opinion. I think the EEG monitoring will help us figure out any actual seizure involvement, hopefully. I do know that it is possible that the episodes that are more seizure-like and not quite fitting into the Complex Migraine symptoms might not be triggered during the monitoring or that epileptic activity might be harder to catch, so we may not have a conclusion on all of this. The important thing is that we get expert eyes on her. Epilepsy experts and hopefully migraine experts who really know the complexity that can exist and don’t try to slap a psychogenic label on her.

And, yes, there is a bit of irony here. Early in this journey, we were sent to a Headache expert who idiotically insisted Em was having migraines. We *knew* she wasn’t. We suspected high ICP and just wanted to try Diamox. Eventually we found a doctor who did prescribe Diamox and it made all the difference. So, for all these years, I have battled constantly to keep the right focus on her headaches.

But the situation has changed. I believe that damn concussion has radically changed the landscape and now we are dealing with a whole slate of new issues. So, if this is migraines, so be it and let’s address it. For that matter, if it is seizures, so be it and let’s address it.

I am hopeful that if we get the right people looking at this, we can figure it out and get her some relief. THAT is the important issue.

Finally, I just want to say how much I appreciate everyone who reads, comments and shares. Not only is it crucial for all of us to know that we are not alone on our journeys, but we all have knowledge that can help someone else who is struggling. My hope has always been that sharing our experiences here would help someone else but we have been so blessed by hearing about other’s experiences as well. We would be lost without you, truly. 🙂

Like this:

I haven’t posted in regards to the important neurology appointment we had this month. Em had been referred to a neurologist at OSU at my request, so we could try to get a diagnosis – either of CRPS or small fiber neuropathy or something. Then the labs done during her LP in January showed some concerning markers for MS and sarcadosis and her local neuro wanted her to see someone more knowledgeable than him. Since we already had this appointment set up, it could serve both purposes. Then, the seizure-like episodes started and added an additional layer of wackiness.

We have learned the hard way not to put too much hope or weight on any single appointment, but I would be lying if we didn’t at least sort of hope deep down that he would be able to give us a diagnosis and a quick solution on the first visit. When he didn’t, it was a little disappointing and overwhelming.

What he did was listen and order a bunch of testing:

He ordered an inpatient stay on the Epilepsy Monitoring Unit to try and figure out what is going on with these episodes. (Side note: after a month and a half of them and a helpful hint from a reader, I think we have a direction to go in. Video EEG monitoring is definitely needed, but will only be a start. More about that soon!) He definitely agreed that we need to rule out all the medical possibilities before jumping to the conclusion they are psychogenic. Don’t have this scheduled yet, but hopefully we can get it done soon, figure out what the heck is going on and stop it.

He ordered a bunch of labs, including mast cell testing. No one, including the allergists Em has seen has been interested in pushing for an actual diagnosis or any treatment other than Zyrtec and Zantac. It would be nice to have someone actually helping with this.

He ordered 3 brain MRIs – one with and without contrast, a MRI Angio and MRI Venogram. I am assuming these will help rule in/ out several things, MS for one. He also was not thrilled with the fact that she has been on Diamox for several years with no evidence and is wanting to make sure there is no structural issue causing her high pressure headaches. He did say he wouldn’t mess with her Diamox but he was clearly skeptical. However, he was shocked that cervical fusions are so common for treating neuro symptoms in EDS. We definitely agreed that it was wise to avoid a fusion if possible so that was a start. Perhaps, with time, I can help him understand the rational of the Driscoll Theory and why we absolutely believe Diamox saved Em’s life. There was just too much to cover in this first visit.

He ordered an nerve conduction study. This is definitely looking at CRPS but her exam also showed some very concerning numbness in her legs. I knew she had numbness but it was far worse than we knew. He pricked her all over with a pin. She mostly could not feel it in large areas on her legs. Like, at all. Her feet and hands are numb but her legs in particular have very little feeling. He seemed concerned. I know I was. So, perhaps we are looking at a neuropathy instead of CRPS? She is having increasing difficulty walking and I have to wonder if this is part of the problem. I don’t know but it is more than a little scary.

He ordered more autonomic testing – another Tilt Table Test and a sweat test and 24 hour Holter Monitoring. He is concerned that much of her problem is autonomic related and seemed to get caught up in the fact that she hasn’t been diagnosed specifically with POTS by the cardiologist. I am honestly not sure what a name does in this case – the cardiologist is treating her for/ as if she has POTS but has said he didn’t want to label it POTS because it is more complicated. So we have just used the term “Autonomic Dysfunction” this whole time. I am conflicted on this – on one hand, I totally agreed with the Autonomic Dysfunction diagnosis as opposed to POTS. On the other hand, I have felt for a long time that they are not doing much for her at the Syncope Clinic. We go once a year and they say, “Increase your Midodrine; see you next year”. So, maybe this reset is what we need. But… I absolutely dread the idea of another Tilt Table Test for her. He says the one she had several years ago wasn’t good enough, that he needs more information. I am not sure I totally see the point, especially considering she will have to be off ALL her pain meds for 3 days. I can’t even say how awful that will be. We are holding off on scheduling this until we talk to him again. We will need some help, some plan to make this happen – a 2 hour car trip is bad enough on a good day, I cannot even fathom how she would manage that trip without pain meds. If it is not absolutely necessary, I can’t put her through that. Yet, I wonder if maybe, with as dire as the situation is, it is necessary to figure out what is going on.

We liked him well enough, but the whole thing was a little overwhelming – he was very clinical, very data oriented and it was both comforting and annoying. Em was frustrated – exhausted, in more pain than she was in to begin with – because while she couldn’t feel most of the pin pricks, it still caused her pain to spike terribly afterwards – and terrified of having to go off her meds. So, she was not exactly rational about the whole thing, not that I blame her.

I eventually came to the realization that he addressed every single issue we brought up with him. He ignored nothing we said and is looking at everything. He was skeptical about CRPS, because it certainly shouldn’t present quite like she does. So it might be a that she has CRPS but the full body pain might be down to Central Sensitization or neuropathy or something else entirely. He seemed to know a little bit about EDS – certainly he knew the connection between EDS and POTS – so we will have to see what level of knowledge he has as time goes by.

The thing that really got me, was that he wanted to see her back in 4 months. When we actually scheduled that appointment, I realized it will be 2 weeks shy of a year since this all started. A year of this torture and no relief in sight. I know the wheels of medicine turn slowly and that we need time to get all of this testing done before we see him again, but knowing we are at least 4 months from any help was absolutely crushing.

So, we are getting all of this testing scheduled and completed and just hunkering down and trying to survive.

I really wish our lives were boring enough to be able to cover events in a single post, but, alas, that is not the case. Have been needing to split posts into multiple parts a lot lately and probably will for some time to come…

When I left off last, Em had had a lumbar puncture and, although I asked for it and the doctor ordered it, the concurrent blood patch was not done. I asked for the blood patch to prevent the very likely problems that were going to occur after the LP – a blood patch simply helps stop a CSF leak from the LP site.

Em did fine after the LP, in fact, her head was pressure free for the first time in a long time. But, unsurprisingly, she started having problems by that evening. Her back was extremely painful and she was enduring a spinal headache – low pressure. She also appeared to have developed another leak in that same ear where the first presumed leak was and that probably added to the low pressure issue. Sitting up was painful, so she laid flat for most of the weekend. We dutifully waited out that time (as ordered) to get through the window until she got to the point where she was either willing to go to the ER or until the doctors would take her seriously.

Monday morning (the 18th) Radiology called to check on her and were concerned with her symptoms and told us to call the neurologist. I did and played phone tag with them for two days. When I finally talked to them, they said to either go to the ER or call the pain doctor. I called the pain doctor and was told he was not in and they had no answers for us.

So, I informed a very reluctant and miserable Emily that we had to go to the ER. Have you ever felt too bad to go to the ER?? (I know many of you know how that is.) Well, that is where Emily was Tuesday afternoon.

When we got to the ER, they took her seriously but likely because we were able to say the neurologist sent us. Which is why I waited until we had their word to go: it can get ugly when we don’t jump through that hoop. Anyway, they took her seriously and quickly got her into a room and got an IV and pain meds started.

Here, it is probably worth mentioning the thought process of the other parental unit in this situation. You know I was angry and frustrated. My hubby was livid that they did not do the blood patch along with the LP and forced us to the ER resulting in yet another bill. So, he was already angry that we had to drag Emily out to the ER, in the bitter cold, on snowy roads, when it likely could have been prevented.

The ER doc walked into this minefield when she came in and said Em needed a blood patch but…

Well, it isn’t considered an emergency procedure so if there were no anesthesiologists available, we would have to just go home and schedule it for the next day. You can imagine how this went over with us.

Fortunately, the anesthesiologist on call was willing to come back in even though she had just gone home. I appreciated that – giving credit where credit is due.

When she got around to doing the procedure, she snapped on the gloves that were in the epidural kit that had been sent up. Unfortunately, those gloves were latex. You may remember that Emily is severely allergic to latex, to the point of if one glove was laying in the corner or if latex had been used in that room for the previous patient, she will have a reaction. Air born latex particles will cause a serious reaction very quickly. I mean, no, she hasn’t died yet from it, but she has taken Benedryl many times and there have been times it has been scary. I would really prefer we not have to prove how bad it is through her death.

So, Em is sitting on the edge of the bed, in pain, half naked, prepped for the procedure, scared out of her mind because she hates needles and vividly remembers how much the LP hurt and has been told this is going to be the same. I am not paying attention to anything but Emily at that point. I am in front of her holding her up, because I know she cannot hold herself up. She looks at me and asks if those gloves are latex. I look over her shoulder and ask, mostly to humor her, because they won’t make that kind of mistake, right?

When I ask about the gloves, the doctor stops in horror and says, oh crap. (Or something like that. My mind was racing at that point but that was the general idea.) And rips them off, apologizing. I look back at Emily and ask if she is ok. She says, “Not really.” So I tell the doctor she needs Benedryl. She assures me she didn’t touch her with the gloves and was horrified to find out that just taking them out of the package was enough to causes a reaction. So, she wraps everything up in the now unusable kit and rushes it out the door and gets the nurse, who brings in Benedryl. (Funny story, she brings it in with a syringe and Emily, in the midst of an allergic reaction, is like, hey, I have my own Benedryl in my bag, no need to give me a shot. I am good. Needless to say she was relieved to find it was going to go directly into her IV, although she was understandably suspicious until it was done.)

At this point, I am past livid. Especially when I realize they hadn’t put an allergy bracelet on her when they checked her in. Would it have made the difference? Honestly, I doubt it. But that isn’t the point, is it? And why does this ill child have to save her own life?

So, they have to order a new kit and leave her sitting, miserable, freezing, scared, and feeling like they had just tried to kill her, for far too long. Finally, they get the new kit and the doctor and the phlebotomist both come in. The phlebotomist sort of joked that the hospital is a latex free facility and they must have found the only pair of latex gloves in the place. I call BS on that because I know better. Their system failed at every step of the way and could have killed her. This is being addressed.

The procedure was, well, not fun. The doctor was trying to get her numb enough to do the procedure but never really succeeded. They took a huge amount of blood (20 mls) pretty quickly, too quickly for her. If I had not been holding her up, she would have been on the floor. You can’t take that amount of blood from a POTS patient without some effect. I thought she needed more fluids afterwards but she just wanted to go home so I didn’t push that issue, although I wish I had.

They gave her time to rest after the procedure and then basically kicked us out. I asked for them to do something for her pain, which was now as bad or worse than it was when we arrived, because we had a 45 minute ride home and she was in agony. They had given her morphine, a couple hours before and were not inclined to give her anything else. They offered her a pain pill, half of what she already takes. The doctor came in and explained since she didn’t know exactly what was going on, she didn’t want to throw narcotics at Emily, which she ironically did anyway. She also implied that we would be violating our contract with the pain doctor if we pushed the issue.

In the end, we had a child who was in more pain than she was to begin with, making a liar out of me. I promised her they would help, that they would treat her pain. Once again, they have bolstered the wall between this ill child and the care she needs by showing me to be a liar and refusing to take her seriously.

These experiences leave us battered and bruised, especially when they should have been prevented. There will be more to this story: we are not going to accept this kind of treatment without making some waves. The latex exposure is huge. Huge. That needs to be addressed. Because my husband works at this hospital, our insurance forces us to do everything possible there. If her specialists at Cincy order blood work or an MRI, can’t do it there, have to go to our local hospital. If she needs to go to the ER, we have to go there. But, they clearly are not providing a safe place for her to be treated. So, yes, this one is a hill worth dying on. They either need to get their act together or allow us to take her somewhere where she will be safe.

We also are going to fight the policy that prevented the blood patch being done along with the LP. I am not stupid – I get that the two procedures are done by different departments and it is complicated, but are you telling me it is impossible to schedule this? Seriously.

At best, this is a situation where the right hand doesn’t know what the left hand is doing. At worst, well, I hate to speculate about profit, but there it is. Then there is the fact that her doctor ordered the blood patch. Ordered it. I was not aware that a doctor’s order could be viewed as a suggestion. If they couldn’t do it, it was on them to contact him so he was aware. They did not call him, neither before or after, I have been told by him personally. Again, their system failed this child at every point.

So, we are beyond angry. There is absolutely no reason any of this needed to happen. To be told they can’t do the blood patch along with the LP and that we just have to go to the ER if there is a problem, only to find out the blood patch is not an emergency procedure, is mind boggling. To be told by the pain doctor’s office they have no answers for us. The whole thing is a nightmare, to be honest.

On the upside, I think the blood patch did help. Her pressure is normalizing (read: getting high again) and she is trying to manage the increasing pressure with her Diamox but not taking too much so it dips down into low pressure. That is a juggling act to be sure. The procedure did not help her back pain, but that is not too surprising. She is still in enormous pain – every movement is painful, but at least the blood patch wasn’t pointless.

We were able to get in to see the neurologist on Friday. That was a minor miracle. And there are other things going on as well, so I will be posting about all of that, trying to catch up. And, with any luck, there will be more to report on how our complaints are received. I don’t want anybody fired, although Emily and I have thought it would be useful to make everyone who had a part in the whole latex portion wear a scarlet “A”. I am thinking it would be an effective deterrent, but I suppose I won’t push that since HR might object.

Honestly, I just want to educate the people providing my daughter’s care. I want to help them be better. I want them to take her problems seriously. I want them to take chronic illness and chronic pain patients seriously. I want policy to change – how many people have suffered and had no recourse like we do? It is not ok. I want to help them offer the care my kid needs. But, there are things that need to change and we are going to do our best to see that happen. In the meantime, Emily is slowly recovering – her physical wounds will heal but her emotional ones will take longer, I think.

Or two or three or…. whatever. As bad as the last 6 months have been, this past week or so has been especially harrowing.

Em hit her head last weekend. Again. Not bad but bad enough to bring on more memory loss and very poor balance. And she had been having this weird thing where she felt like her throat was collapsing, which was more than a little freaky. Along with the latest head injury came worsening headaches and a very unstable neck. Just like 5 years ago, she was having all kinds of problems and I started assuming many of them were from cervical instability. And that was not a pleasant thought.

The concussion doc was willing to see her, but we agreed that there was nothing he was going to be able to do – time to heal and protecting her head from further injury is what was needed. So, we will see him at her follow up February 5th and wait and see. He is a phone call away.

We decided that some blood work – particularly to check her CO2 level but also a couple other things – would be a good idea. So I called the Neurologist to ask for that. I was not surprised when the answer I got back was that they were ordering blood work and a lumbar puncture.

For more than 4 years, we have avoided lumbar punctures. Not only was I aware that they might not be completely conclusive regarding high pressure but EDS folks are delicate enough that even this relatively easy procedure should not be done lightly. Once I found the Driscoll Theory, I was 100% comfortable doing a trial of Diamox instead of an LP. If Em responded to Diamox, then it would logically follow that her headache was the result of high pressure. We found one doc who was willing to try and the rest is history. The headache she had had for 2 years was gone in a few hours after that first dose. For almost 4 and a half years, we have managed her headache with Diamox, eventually having to increase her dose and taking it 3 times a day rather than once, and we have managed her CO2 level (which allows the Diamox to work) with baking soda tablets. We had tried to skate around the issue with new docs, endured some eye rolling when we admitted she was being treated for high pressure but had never been actually tested for it and just generally crossed our fingers hoping no one would push the issue. It was all fine for 4 years.

But now, clearly something had changed. My suspicion was it was the head injuries but she probably had a CSF leak (in her ear) in December before the concussion, so maybe the headache is coincidental or cumulative or something, but the bottom line is what we had been doing was no longer working.

In my mind, there were 3 possibilities: she needed more Diamox to control the high pressure, her CO2 level was low which would mean she needed more baking soda tablets to bring it up so the Diamox would work, or there was something else entirely going on, like cervical instability. At any rate, I knew in my heart the time for an LP had come. So, I agreed but asked for a couple things to hopefully make the procedure easier and more successful. I wanted them to use an atraumatic needle, to minimize damage and leaking. And I asked that they schedule a blood patch to be done immediately after the LP, instead of waiting until she had problems. Because, let’s be real, there was a nearly 100% chance she was going to have problems.

The doctor’s office ordered both without issue, so I was happy.

A nurse from Radiology called to pre-register her for the procedure and asked a ton of questions about EDS and her medical issues. She was awesome. She promised to check into the special needle – she had never heard of such a thing before – and did say she wasn’t sure about doing the blood patch because they normally don’t do them but that she would ask the doctor and if she could do it, she would because Em is clearly a special case. So I was content that I had done everything I could to make the LP as safe and easy as possible. When I heard nothing back, I assumed the blood patch was a go.

Em was not thrilled – anything involving a needle is not her idea of fun – but as we waited for Friday I started thinking it couldn’t come quickly enough. Her headache was a 12 (on a scale of 1-10) when her Diamox wore off and a 9.5 when it was working. Clearly there was a problem: she was miserable. We got the blood work that I asked for done and were able to slide in blood work they needed before the LP at the same time, so we minimized the needles for her. When even a simple blood draw is challenging, is it any wonder we were nervous about a lumbar puncture?

We get there on Friday morning and once she is in the room and has been given some Zanax to help relax her, I find out that they will not be doing the blood patch. LPs are done by Radiology and blood patches are done by Anesthesiology.

“Sorry, I don’t know why you were ever told it was possible. It is our policy to not do them here”,” the nurse said. “If she has problems, you will need to call your Pain Management doctor or go to the ER.”

Now, at this point, I am starting to fume. No one has called me to tell me this and I was given the impression it was happening. So, when she said that, I just gave her a look. The Look. Which she correctly interpreted. She sheepishly acknowledged that, since it would be over the weekend, we would, in fact, not be able to get a hold of the pain doc and that we would just need to head to the ER. I told her I understood this was not her fault, but that we had specifically asked for the blood patch to avoid almost certain problems that would arise due to EDS, avoid a trip to the ER, avoid being charged for the trip to the ER, avoid causing Emily more pain and suffering, etc.

So then I, huffily, I am sure, asked about the needle. She told me that they didn’t have the atraumatic type and the doctor didn’t think it would make a difference anyway. By this time I am livid…. Halfway tempted to tell them to stop and refer us to somewhere else that can meet Emily’s needs and I told the nurse so. And, that with all due respect, I disagreed with the doctor’s opinion and that I did indeed believe that the atraumatic needle is necessary. I was very unhappy and I wanted to walk away, but, in my heart, I knew she needed this procedure done and that it could wait another week or two to reschedule it.

Then the doctor comes in and introduces herself and tells me she will be using the special needle. I immediately, and understandably, assume that I am being lied to because I have just been told otherwise. But again, I feel trapped – not because it would be an inconvenience to stop the procedure, but by sheer need. And I did not believe I was going to get a straight answer even if I made an issue of being lied to. Then I had to leave my medically complex child on the table with people who I believed were lying to me. This may be the way things are done, but this did not help my mental state.

My hubby popped down (he works at the hospital) to check on us and he found a very unhappy mama fuming in the waiting room. He had to get back to work before she was done so I was alone when the doctor came out to talk to me.

Turns out she wasn’t lying after all – she did actually use the atraumatic needle. She said she didn’t like it, it felt weird going in, although it worked fine. I don’t know if the nurse had told her I was mad about it and she decided to use it or if she was planning on using it all along, but… whatever. The nurse later apologized for telling me wrong, implying there was a lack of communication somewhere along the line. I tend to think the doctor changed her plan in light of my anger but I suppose I will never know.

She had to lie flat in recovery for an hour, but, and this is the third EDS specific request I made, I told them she was going to need to be in recovery for longer than normal. No matter how many times I said this, they still started pushing her out the door. At about an hour, they had her sit up – and she found that her head actually felt normal for the first time in a long time. Her head felt better after the LP than it did before. Her back, not so much. In the end, I could not fight both the nurse and Emily who said emphatically, “I am starving. Get me out of here and feed me.”

So we left, I fed her and she went home to lay flat for the rest of the day.

Anyway, after all was said and done, we did fortunately get some valuable information from the LP. You may have already guessed that by her headache being relieved by the LP that she did indeed have some high pressure going on. Her opening pressure was 20.2. The doctor told Emily that normal should be between 10 and 15; I read the range is 7 – 18. Either way, Em had high pressure, although she is on Diamox three times a day. She had taken her morning dose 2 hours before the LP, which means it basically should have been reaching its peak effectiveness when the LP was done.

We have our proof as far as I am concerned. I would be fascinated to know what her pressure would have been without Diamox – not that I am willing to find out, mind you. But, my guess is it would be quite high. In fact, the symptoms that had been plaguing her: headache, unstable neck, blurred vision, light/ sound sensitivity, even that issue with her airway, all improved dramatically when the pressure was relieved. Exactly like it did September 19, 2012 when she took her first dose of Diamox.

Once we knew that there absolutely was high pressure causing the headache, the burning question became: is her pressure high because her dose needs increased or because her CO2 is low, thus making the Diamox ineffective? Hubby was able to call that afternoon and get her CO2 level – 26. It needs to be 21 or higher. This clearly is not the issue. We don’t have all of her bloodwork back yet, but that was the important one at the time.

Oddly, she had what we think might have been another CSF leak Saturday night, in her bad ear. She was laying on the side with the ear that has been problematic for months now and suddenly said that her ear was wet. I declined sticking my finger in her ear like she asked me to but her hair was damp where she had been laying. And she got a tissue and, after laying on it for a while, it was damp. But, it cleared up quickly, we think, and we saw no more signs of a leak.

However, this leak may have added to the problems that were to follow. If that sounds ominous to you, you are on the right track.

Part 2, in which we unsurprisingly and very predictably end up in the ER and have LOADS of fun, is coming up soon…

As we waited to get to the concussion experts – and fortunately we did not have to wait long – Emily’s concussion symptoms remained concerning. She is having trouble reading and comprehending, she struggled to write – to spell and remember the mechanics of language. As we passed the 3 week mark of her head injury, she was not getting better and, while she was perhaps not getting worse, more deficits were being revealed. I am not sure we even know the extent of her deficits at this point. When she is searching for a specific word, we go through a very entertaining version of verbal charades where she tries to make me understand what she is trying to say, with sometimes hard to follow logic and obscure literary references. Yesterday, she tried to use a story from the Little House on the Prairie books, to get to the word “fraternize”. We got there but, man, it was a circuitous journey! As always, we are still able to laugh at ourselves and the situation.

At the Concussion Clinic, we saw a rehab doctor, a neurologist and a doctor who I believe was a fellow, which is very common in our experience at Children’s. The exam revealed that her short term memory is crap, to put it plainly. She was given 3 words to remember. Maybe 5 minutes later she was able, with some effort, to pull up the first one, “green”. She was eventually able to guess the second word, “liberty” when given a prompt that it was another word for freedom. She could not recall the third, “automobile” even when given a prompt that it was a word for getting around. She guessed “car”, was told it was something like that and guessed “bus”. She remembered the word when he said it. She also took a fair amount of time to be able to say when her birthday is, which is not normal and is something she answers every single time she is at the doctor.

Her balance is off – she struggled to walk a straight line. Her eyes are not right – she either showed nystagmus or saccadic oscillations/ interruptions. (Her eyes were jerky when tracking side to side; I was able to clearly see them from where I was sitting. There was some discussion about this – I was familiar with nystagmus but had to look up “saccadic”. I was able to do so, even though it was a totally unfamiliar word, because we had a funny moment when Emily thought the doctor called her “psychotic”, “saccadic” does sound similar. He laughed and told her he would never call her psychotic to her face. We liked him quite a bit. 🙂 So, I was able to find what they said, I just don’t know the significance of it.)

So, they all agree that she did have a concussion and she has post concussive symptoms. Likely the fact that she (probably) had a CSF leak made her brain extra vulnerable to injury at the time because there was less cushion to protect it. They were concerned with the Diamox contributing to her CSF volume, but she was not taking it at the time of her injury. It was a day or two later that she started taking it again. They have referred us to speech therapy where they will assess her and do cognitive therapy to help get her brain back in shape. We are going to do that at our local hospital and they were fine with that. We will go back in a month and they will do neuropsych testing to further evaluate her, particularly if therapy is not helping as much or as quickly as we would like.

They were understandably concerned with the number of meds she is on. Frankly, they can join the club. Of course we are concerned and, yet, this is what has made her functional and removing any of them seems insane. They didn’t push that and said they would not mess with her meds since she has other docs overseeing them. I was thankful for that. Very thankful. Particularly that they left the Diamox alone.

The neurologist, through no fault of her own, should have come with a trigger warning. She was nice enough but she had the same basic advice that we got at the headache clinic at Cincy 4 years ago: eat your vegetables and drink your milk. Once again, her point is absolutely correct, as it was 4 years ago. Eating well and nourishing your body is vital to wellness. Unfortunately, that gets a little complex when one has a handful of medical conditions that make eating/digesting hard. Furthermore, when we mention that Emily is lactose intolerant and that detail is ignored because it goes against their standard protocol, it makes her a little tiny bit furious. That day, she was exhausted, hurting, scared and now this “chick” is telling her to drink milk. That said chick was a little heavy on barking orders and light on (visible) compassion did not help. Em has a, ahem, less than favorable view of her but, I know she was just doing her job. And we have the same goal – to help Em. Once they left Em let loose about her and didn’t even laugh at my favorite medical joke: How are God and a neurologist different? God doesn’t think he is a neurologist. She did say she wanted to go give her neurologist at home a hug for not being a jerk and taking good care of her. And, presumably not telling her to drink milk.

We were suitably impressed with them over all; I feel like she is in good hands. However, I am not sure they truly understand her complexity. My guess is they don’t see a ton of (diagnosed) EDS patients in their clinic and so would have little reason to be familiar with it. I mean, you can understand the general mechanics of connective tissue and not fully grasp the entirety of what EDS will do to a person or how the simplest thing is a huge challenge.

They knew her shoulder was bad, she was wearing a sling – the MRI report was in her records, but it was like they didn’t quite grasp how unstable it is, how much it hurts and how much the CRPS pain affects her. The Rehab Doc was concerned over the tightness/knots in her neck and shoulders, because that typically is seen with concussions and contributes to the headache. What we were not able to really communicate is that that is not new – that is her baseline. And when we relieve those knots or muscle spasms, the adjoining joints often are unstabilized. Could that tension be contributing to her “concussion headache”? Sure. Is it going to be easily managed? Probably not. He wanted her to do a few stretches, some of which she cannot do because of her shoulder. In the end, he told us to talk to her PT and have him work on addressing it. The PT was incredulous that the doc would have her doing anything with her hypermobile neck and said, “Look, I don’t want to tell you to he is wrong, but don’t do any of that. We have to protect your neck. And you can tell him I said so.”

Once again, we are trying to navigate this ocean of conflicting advice. We just do our own research, try to know what we need to know and make an educated decision. We tend to go with the advice that makes the most sense at any given time and aligns the most with our understanding of all of the pieces of the puzzle. We also tend to go with the advice from the professionals who know her best.

So, the situation is at least as serious as I feared and perhaps more. It was really hard to see her struggle and see the fear and frustration on her face when asked to do a task she couldn’t do. But, concussions can take months to recover from and it is entirely possible that we will see her brain heal spontaneously over the next few weeks. Therapy will help and my husband, as always, has handpicked the best therapist for Em and her situation. We are optimistic and hopeful that she will get better in the near future.

Continued in Part Three: Shoulder…. Because that is a thing. Just not quite as big a thing as her brain.

This post is long overdue (and I apologize for that!) but I said I would post when the updated version of the Driscoll Theory was available so, here goes! (Better late than never!)

You can go to Dr. Diana’s website and purchase the ebook. The $19.99 cost will help fund continuing research (and there is more on the horizon, trust me!)

From Dr. D:This book is written by doctors and patients who live with EDS/POTS every day. Revealing the cause of “EDS/POTS” results in a new treatment paradigm.All proceeds from this book will be used to fund further research. Gentle hugs to you all…

I was privileged to help out a bit as Diana was working on this revision, as well as to contribute a piece about our experiences with the Driscoll Theory. Our story (Emily’s Story) is in the book (in Chapter 10), as are touching stories from other patients who have been helped by Dr. Diana’s work. In addition, there are chapters from physicians who have both personal and professional knowledge to share. Of course, POTS, intracranial pressure, mast cells, vascular abnormalities and more are explained and discussed.

Pop over to prettyill.com and grab your copy of the new Driscoll Theory. (I have it on my Kindle, for late night references and doctor visits, as well as my computer, so it is always available!)

Feel free to come back here and comment, once you have read it! If you have questions, either comment here or go to the Ask a Question page and comment there. If I can answer your question, I will. If I can’t, I will pass it along to Dr. D and have her answer you.

Having the Driscoll Theory in print once again is the next step to ensuring the paradigm shift that started more than 3 years ago becomes a standard of care for EDSers. So, it is important that we share this with everyone who could benefit!

Finally, I would like to thank Dr. Diana for her work in making her theory available for all of those who desperately need a bit of hope. I think we all sometimes forget that Diana is just like all the other EDSers out there – she has her own medical challenges to face and she has a family to care for – so that makes this project, that she worked so hard on, even more special. Thank You Dr. D!

Ok, I have tried to keep the ranting to a minimum – there are better things to offer the world than me being mad about this, that or the other thing. However, I need to vent about this somewhat inconsequential thing because it really rubbed me the wrong way. Still mad a couple weeks later…

Y’all know that Em is on Diamox via the Driscoll Theory. September will mark 3 years on it and it literally changed her life. Y’all also [probably, hopefully] know that Diamox affects the CO2 levels: to combat the level dropping and to ensure that the Diamox will continue working as it is intended, one needs to take baking soda. (Not gonna get into the science of it, but you can look it up.) The easiest way to take baking soda is to just purchase some tablets – you can make your own capsules but it is kind of a pain in the butt.

We got Em on baking soda, unfortunately after her CO2 levels dropped instead of being preemptive about it, several months after starting on the Diamox. After struggling with making capsules for a while, I eventually caved and went to Walgreens to procure some. Walked up to the pharmacy counter and said, ‘Do you have baking soda tablets?’ The nice young man hemmed and hawed for a bit, trying to figure out exactly what I needed, then said they did and asked how many I wanted. No prescription needed, obviously.

You can buy the darn things online. Seriously. Sodium Bicarbonate is not exactly a restricted substance.

So, every month or so for the last 2 years, I have popped into Walgreens and bought 200 baking soda tablets without any problem whatsoever.

Until a couple weeks ago, when I came up against the New Sheriff in Walgreens Town (aka the new pharmacist). Who adamantly refused to fill my request because I did not have a prescription. For BAKING SODA TABLETS. My informing her that I had purchased said tablets for over two years at that very pharmacy, not only without a prescription but without EVER being asked for a prescription, made no impact on her stance. She said she could not open the bottle of baking soda tablets without a prescription – it was against regulations. So, either she is full of it or the handful of pharmacists who have filled my order for the last two years were breaking the law. (I will give you three guesses as to my opinion about that!)

She magnanimously offered to call the doctor and get a prescription for it – at 7:45 on a Thursday evening. I more-or-less (possibly less) politely informed her that I really doubted they would be in to take her call. I told her I was leaving on vacation and my daughter had to have them before I left. She offered to give me enough to get through the night. I refrained from pointing out that she certainly wouldn’t do that if a customer said, ‘golly, I don’t have enough Oxycontin to get me through tonight – can you give me just a few to get me through?” So, if she was going to open the 1,000 count bottle and break the “rules” to give me enough baking soda tablets to “hold me” until I could get a prescription, why on EARTH could she not just give me the 200 I was asking for?

Ugh.

We had priced the tablets at the hospital pharmacy when we first started getting them and found out it was much cheaper to get them at Walgreens, but when the New Sheriff in Walgreens Town refused to provide them, we had to go through the Reid pharmacy. Over $5 for the equivalent of 50 tablets (they only had 325 mg tabs in 100 count bottle), not even enough to get Em through a whole week. And, being half the size meant she got to take 6 pills instead of 3, which she was thrilled about.

All because some crazy lady decided she couldn’t sell me baking soda tablets. Seriously. Makes me want to pull my hair out. Or, purchase a capsule filling kit, take it with me to Walgreens, purchase a box of baking soda at the pharmacy, then open it right on her pharmacy counter and proceed to make a few dozen baking soda capsule in front of her. Pointing out of course, that this is the controlled substance she wouldn’t sell me.

Sometimes, on the journey of living with chronic illness, it is the little things that really get to you. Little things like baking soda tablets become really big things. We juggle so much worry and fear and pain and uncertainty, frankly, with a lot of acceptance and, most of the time, dignity. But, to have to fight and claw over something as small, yet vital, as baking soda tablets is just too much.

We have it worked out now and I know I really should get over it. My mom ordered (without a prescription, mind you) a couple 1,000 count bottles so we won’t have to worry about it for months and that is a nice thing. Walgreens has lost my rather substantial baking soda tablet business, so if they go bankrupt and have to close their doors, you will know why.

But, although I know it is all good and I should let it go, I still have this urge to prove my point to the lady at Walgreens…

I wonder if a baking soda volcano on her pharmacy counter would make an impact?

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Thanks for visiting my blog where I chronicle 'Our Life with Ehlers Danlos Syndrome'. I am sharing my family's journey with EDS (and everything that goes along with it) in the hope that our experiences will help someone else.

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